新生儿高铁血红蛋白血症研究进展

Progress in neonatal methemoglobinemia

新生儿高铁血红蛋白血症是一种以青紫和低氧血症为临床表现的罕见疾病。细胞色素b5还原酶异常以及血红蛋白M症是引起遗传性高铁血红蛋白血症的主要原因。接触利多卡因、亚硝酸盐等物质则可能诱发获得性高铁血红蛋白血症。血气分析是高铁血红蛋白简便的检测方法。高铁血红蛋白血症与新生儿腹泻、酸中毒、晚发型败血症等多种疾病相关。亚甲蓝是治疗高铁血红蛋白血症的常用药物, 维生素C、N-乙酰半胱氨酸、维生素B2在治疗上可能也有一定疗效。该文对新生儿高铁血红蛋白血症的研究进展做一综述。

Neonatal methemoglobinemia is a rare disorder characterized by cyanosis and hypoxemia,which could be caused congenitally by cytochrome b5 reductase enzyme deficiency or hemoglobin M disease,and could be acquired by the exposure to lidocaine,nitrites and other drugs.Blood gas analysis is a simple and accessible way to detect methomoglobin.Methemoglobinemia is related to numerous diseases in neonates,including diarrhea,acidosis,late-onset sepsis.Methylene blue is an effective drug for decreasing MetHb levels.Other therapeutic options,such as vitamin C,N-acetylcysteine and vitamin B2,could also be useful.This article reviews the progress of neonatal methemoglobinemia.